Clinical and Pathological Features of Primary Neuroectodermal Tumor/Ewing Sarcoma of the Kidney

Risi, Emanuela; Iacovelli, Roberto; Altavilla, Amelia; Adami, Daniele; Palazzo, Antonella; Mandato, Claudia; Trenta, Patrizia; Cortesi, Enrico

doi:10.1016/j.urology.2013.04.015

Cited by 67 publications

(96 citation statements)

References 16 publications

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“…In addition, IHC markers, including vimentin, NSE and S-100, may aid in the diagnosis of rPNET and differentiation from other tumor types; however, they are not pathognomonic. Molecular diagnostic markers, such as friend leukemia integration 1 (FLI-1) and WT-1, were found to be relatively specific in terms of the diagnosis of rPNET, and a previous study reported that 60% of rPNETs were positive for FLI-1 expression by IHC (17), indicating that this marker may aid in the differentiation of rPNET from other types of renal tumor. Within the cohort of the present study, the tumors of 37.5% of cases had a specific histological structure called 'Homer-Wright rosette', and IHC analysis revealed that 65% of cases were positive for CD99, vimentin and NSE.…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

et al. 2018

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Abstract. Primitive neuroectodermal tumor (PNET) rarely occurs as a primary renal neoplasm. Renal (r)PNET is a rare but aggressive neoplasm with poor prognosis; the majority of patients are diagnosed as advanced stage at presentation and face a worse prognosis than patients with localized disease. The present study describes the diagnosis and management of eight cases of rPNET at an advanced stage, who were treated at two institutions [Lingnan Hospital (branch of The Third Affiliated Hospital) and the Cancer Center of Sun Yat-sen University, Guangzhou], from December 2004 to January 2013. The clinical and pathological results of all patients were retrospectively obtained. Kaplan-Meier analysis was performed to estimate patient survival. The study cohort comprised five males and three females. Radical nephrectomy was performed in seven cases, while the remaining case only received needle biopsy of the tumor. Five cases received adjuvant chemotherapy, while three received no further treatment after surgery. Of note, one case received cytokine-induced killer (CIK) cell immunotherapy combined with surgery and chemotherapy. The overall median survival was 20 months with a 3-year survival rate of 25%. The overall survival of the four patients who received adjuvant chemotherapy following surgery was 36 months, compared with 10 months in the three patients without further treatment. The patient who received CIK cell immunotherapy survived for 20 months. Based on the observations of the present and previous studies, surgical excision and chemotherapy are recommended for treating rPNET at advanced stage. Furthermore, the present study was the first to report on CIK cell immunotherapy for a patient with rPNET, indicating that it may be a promising optional treatment. However, further studies are required to validate the benefit of CIK cells and to establish an appropriate immunotherapy protocol.

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Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

et al. 2018

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“…As in our case, it has a predilection for male gender and in contrast to the clear cell, papillary and chromophobe types of renal cell carcinomas, it occurs more in a younger population [2]. Clinically, the most common presenting symptom is pain, followed by hematuria and renal mass [5]. Local recurrences and early metastatic disease are the causes of poor prognosis.…”

Section: Discussionmentioning

confidence: 67%

CT and MRI Findings in a Rare Case of Renal Primitive Neuroectodermal Tumor

et al. 2016

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SummaryBackgroundPrimary renal primitive neuroectodermal tumor/extraskeletal Ewing’s sarcoma (PNET/EES) is a very rare renal tumor.Case ReportWe report a case of primary renal PNET/EES of the kidney in an adult patient and describe its computed tomography and magnetic resonance imaging findings, including diffusion weighted images along with a review of the current medical literature.ConclusionsAlthough very rare, a relatively large renal mass which shows very infiltrative growth pattern on CT and MR imaging and striking diffusion restriction should raise the suspicion of a renal primitive neuroectodermal tumor, in a young adult.

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“…12,13 The presenting symptoms of renal PNET are nonspecific, including flank pain, hematuria symptoms related to genitourinary infections. 14–16 Laboratory parameters are mostly with in normal range except altered level of lactate dehydrogenase and neuron specific enolase in several reports. 13,16 CT images of renal PNET are also without characteristic signs, commonly revealing solitary, large, ill-defined, irregular, or lobulated heterogeneous mass.…”

Section: Discussionmentioning

confidence: 98%

Renal Primitive Neuroectodermal Tumor

Yang

Zhou

et al. 2015

Medicine

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Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor and typically arises from bone or soft tissue in adolescents and young adults. Renal PNET is extraordinarily rare and exhibits highly aggressive biological behavior with poor prognosis.We present here a new case of renal PNET in a 31-year-old female. The patients were referred to our hospital because of left flank pain with nausea and vomiting for 1 week. A computed tomography scan revealed a 14.7 × 12.7 cm well-defined, unevenly mass lesion with both solid and cystic components and the tumor was not enhanced uniformly.A preoperative diagnosis of cystic renal cell carcinoma and urinary tract infection was made. The patient undergone anti-inflammatory therapy followed by a left radical nephrectomy. Taken with morphological pattern and immunohistochemical markers, a diagnosis of renal PNET was made. Two cycles of combined chemotherapy were executed. At the 14-month follow-up, no evidence of metastasis or recurrence was indicated.This case reminds clinicians that for adolescents and young adults with a suspicious renal mass, a diagnosis of renal PNET should be always considered. An initial surgery followed by radiotherapy and chemotherapy is suggested for the therapeutic management.

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Clinical and Pathological Features of Primary Neuroectodermal Tumor/Ewing Sarcoma of the Kidney

Cited by 67 publications

References 16 publications

Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

Primitive neuroectodermal tumor of the kidney at the advanced stage: A case series of eight Chinese patients

CT and MRI Findings in a Rare Case of Renal Primitive Neuroectodermal Tumor

Renal Primitive Neuroectodermal Tumor

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